Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review

Our plain-English summary of Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review, by M. Al-Shraim, B. Mahboub, P.C. Neligan, et al (Journal of Clinical Pathology, 2005).

The study

• Authors: M. Al-Shraim, B. Mahboub, P.C. Neligan, D. Chamberlain, D. Ghazarian
• Journal: Journal of Clinical Pathology
• Published: 2005;58(1):107-9
• Type of article: Case report, Review
• Cited by: 38 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour of intermediate behaviour. It can arise from various sites including the liver, spleen, pleura, or lung. Cutaneous EHE can be primary or secondary. This report describes the case of a 51 year old man who presented with a history of dry cough, shortness of breath, and pleural effusion, and who developed two cutaneous nodules in the anterior abdominal wall a few weeks later. He had a previous history of asbestos exposure. Computed tomography scan showed a left sided pleural effusion and nodular pleural mass. Histology of both the pleural and cutaneous lesions was compatible with EHE. Electron microscopic examination demonstrated the presence of Weibel-Palade bodies. The patient underwent elliptical excision of the metastatic cutaneous nodules after decortication of the primary pleural tumour and adjuvant treatment. A few reports have described metastasis of intrathoracic EHE to the skin. Despite treatment with interferon, the patient developed more cutaneous lesions two years after the initial diagnosis. Even though the tumour has the classic light histological and ultrastructural features of EHE, it behaved in an aggressive manner.

In plain English

This case report described one 51-year-old man treated in Toronto, Canada. He had a dry cough, breathlessness and fluid around the left lung. He had been exposed to asbestos for two to three months about 25 years earlier. Pleural biopsies were first read as malignant mesothelioma, and he received one cycle of chemotherapy. A pulmonary pathologist then revised the diagnosis to epithelioid haemangioendothelioma (EHE), a rare blood-vessel tumour. A CT scan showed a pleural mass up to 6.2 cm. The tumour stained for blood-vessel markers but not for mesothelium-associated markers, and electron microscopy showed Weibel-Palade bodies. After surgery, and after starting interferon, he developed nodules under the skin of his abdomen with identical features. These were interpreted as spread from the pleural tumour. The remaining chest tumour did not change size over two years, but more skin nodules later appeared on his trunk. The authors noted his asbestos exposure was short and imaging showed no pleural plaques. They concluded that EHE can mimic mesothelioma and should be suspected in cases of pleural mesothelioma.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report of a single patient, with a short discussion of earlier published reports
• Who was studied: One 51-year-old man, referred to Princess Margaret Hospital, Toronto, Canada; dates of care not stated
• Exposure: Asbestos exposure lasting two to three months, about 25 years before presentation; fiber type and job not stated
• Main results:
  • Pleural biopsies were first interpreted at a regional hospital as malignant mesothelioma, undifferentiated, small cell type
  • On review, a pulmonary pathologist revised the diagnosis to epithelioid haemangioendothelioma (EHE)
  • CT showed pleural thickening forming a nodular mass up to 6.2 cm in greatest dimension
  • The tumour cells were immunoreactive for vascular markers including CD31, factor VIII and CD34, and stained negatively for mesothelium associated markers
  • The EHE diagnosis was confirmed by finding Weibel-Palade bodies on electron microscopy
  • Subcutaneous nodules on the anterior abdominal wall, which appeared a few weeks after interferon was started, had histology identical to the pleural tumour
  • Serial chest CT scans showed no change in the size of the residual tumour over two years of follow-up
  • More recently the patient developed more cutaneous nodules on his trunk
• The authors' conclusion: The authors concluded that pleural EHE can mimic malignant mesothelioma and should be suspected in any pleural mesothelioma lesion, because management and prognosis differ. They also concluded that this tumour behaved aggressively despite its classic EHE features.
• Limitations the authors state:
  • The authors noted that the patient's asbestos exposure was short and that he had no radiological features such as pleural plaques. They said a link between asbestos and pleural EHE had been suggested in only one earlier series of three patients.
  • The authors stated that the meaning of the tumour's monoclonal CEA staining for prognosis in EHE is uncertain and needs further investigation

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Pleura, Pleural Effusion, Asbestos Exposure, Histology, Metastasis, Intrathoracic.

Related studies

• Primary epithelioid haemangioendothelioma of the peritoneum: an unusual mimic of diffuse malignant mesothelioma — R.L. Attanoos, N.S. Dallimore, et al. (1997)
• Spontaneous bilateral haemothorax as presentation of primary pleural epithelioid haemangioendothelioma — L. Ferreiro, E. San-José, et al. (2017)
• Epithelioid malignant mesothelioma metastatic to the skin: A case report and review of the literature — R.E. Ward, S.A. Ali, et al. (2017)
• Peritoneal malignant mesothelioma with multiple distant skin metastases — N.G. Ordóñez, J.L. Smith (1983)
• Skin metastasis of pleural epithelioid malignant mesothelioma — T. Terada (2011)
• CAMTA1 immunostaining is not useful in differentiating epithelioid hemangioendothelioma from its potential mimickers — Z. Yusıflı, K. Kösemehmetoğlu (2014)

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